By Peter L. Greenberg
This publication describes the medical type, underlying organic mechanisms and therapy of the myelodysplastic syndromes. Authored via foreign specialists, the chapters conceal all facets of the myelodysplastic syndromes, from an in-depth research of the multifactorial nature of this disorder, together with an overview of stromal, immunological and stem telephone abnormalities, to a assessment of contemporary molecular and cytogenetic discoveries and insights. This booklet might be a necessary source to clinicians and researchers who desire to study extra approximately myelodysplastic syndromes.
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Extra info for Myelodysplastic Syndromes: Clinical and Biological Advances
In addition, unrelated to the classification per se, there was some variability in the application of definitional criteria for myelodysplasia by different observers. Nevertheless, the FAB classification has served Myelodysplastic Syndromes: Clinical and Biological Advances, ed. Peter L. Greenberg. Published by Cambridge University Press. C Cambridge University Press 2006. 33 34 Richard D. 1 French–American–British classification of myelodysplastic syndromes2 Refractory anemia (RA) Anemia Usually no blasts in peripheral blood; if present, < 1% Marrow myeloblasts (types I and II) < 5% Erythroid hyperplasia and/or dyserythropoiesis Granulocytes and megakaryocytes almost always normal Rarely patients with isolated neutropenia and/or thrombocytopenia but no anemia may be included in this category Refractory anemia with ringed sideroblasts (RARS) Findings similar to refractory anemia with the addition of ≥ 15% ringed sideroblasts in the marrow Possible dimorphic erythrocytes in blood Refractory anemia with excess of blasts (RAEB) Always some degree of cytopenia involving two or more myeloid cell lineages Peripheral blood shows conspicuous abnormalities in all three myeloid cell lineages Circulating myeloblasts < 5% Marrow myeloblasts (types I and II) 5–20% Varying degrees of either granulocytic or erythroid hyperplasia Always evidence of dysgranulopoiesis, dyserythropoiesis, and/or dysmegakaryocytopoeisis Refractory anemia with excess of blasts in transformation (RAEB-T) Cytopenias in patients, generally with an indolent course Hematologic features similar to RAEB with addition of one or more of the following: (1) 5–30% myeloblasts (types I and II) in the peripheral blood (2) Marrow myeloblasts (types I and II) 20–30% (3) Presence of Auer rods in granulocytic precursors in patients with < 30% blasts in blood and/or marrow Chronic myelomonocytic leukemia Defining criterion presence of monocytosis (> 1 × 109 /l) Often associated with increase in mature granulocytes < 5% myeloblasts in peripheral blood Bone marrow shows significant increase in monocyte precursors Percent of marrow myeloblasts 1–20% 35 Morphologic classifications of MDS as a valuable tool for classifying patients with myelodysplastic disorders and as the classification system of MDS in several clinical trials.
Leukemia, 9, 370. 97. , Messa, F. et al. (2003). Significant correlation between the degree of WT1 expression and the International Prognostic Scoring System Score in patients with myelodysplastic syndromes. J. Clin. , 21, 1988–95. 98. , Bertheas, M. F. et al. (2002). Expression and prognostic significance of Bcl-2 family proteins in myelodysplastic syndromes. Am. J. , 70, 115–25. 32 Peter L. Greenberg 99. , Rott, L. (1996). Altered oncogene expression and apoptosis in myelodysplastic syndrome marrow cells.
Am. J. Clin. , 101, 123. 10. , Van den Berghe, H. et al. (1975). A new hematological syndrome with a distinct karyotype: the 5q− chromosome. Blood, 46, 519. 11. , Dewald, G. W. et al. (1993). The 5q− syndrome: a single institution study of 43 consecutive patients. Blood, 81, 1040. 12. , Paulau, N. M. et al. (1991). Leukemic transformation in patients with 5q− and additional abnormalities. Haematologica, 76, 363. 13. , Shen, Y. et al. (2002). Myelodysplastic syndrome is not merely “preleukemia”.